A rare presentation of systemic lupus erythematosus with lupus hepatitis

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem inflammation and the presence of circulating autoantibodies directed against self-antigens. Prevalence of SLE in children and adolescents is 1-6 per 100,000 population. Liver dysfunction occurs in approximately in 50-60% of the patients with SLE.And the incidence of lupus hepatitis in diagnosed SLE patients is 9.3%. We are reporting a case of 11-year-old female child who presented with fever, skin rashes, oral ulcers, irritability and positive antinuclear antibody (ANA) with deranged liver function test (LFT) in the form of elevated transaminases, bilirubin level and altered coagulation profile. Hepatitis serology was negative, with low C3 levels, diagnosed as SLE with lupus hepatitis with lupus nephritis stage IV with psychosis. Clinical improvement along with improvement in terms of laboratory findings was seen on corticosteroids therapy. It is important to differentiate lupus hepatitis from autoimmune hepatitis (AIH) as AIH presents similar to lupus hepatitis, has poor prognosis. While lupus hepatitis being rare cause for mortality in patients with SLE has good response with early intervention with corticosteroids therapy.

Liver involvement in patients with systemic lupus erythematosus

Liver involvement in systemic lupus erythematosus is common and in most cases clinical course is asymptomatic, that makes diagnosis difficult. Determination of the cause of the liver involvement is important to select treatment and to evaluate the prognosis of the disease.The aim of the research was to characterize the clinical features of liver involvement in patients with systemic lupus erythematosus and identify the most significant clinical and laboratory parameters for the differential diagnosis of lupus hepatitis.Materials and methods. The study included 313 patients with systemic lupus erythematosus observed in the E.M. Tareev Clinic of Rheumatology, Internal Medicine and Occupational Diseases of I.M. Sechenov First Moscow State Medical University (Sechenov University) in the period from 2001 to 2019. The verification of diagnosis of systemic lupus erythematosus was based on the criteria of the American College of Rheumatology (1997). Patients examination included complete blood count, biochemical and immunological blood tests and an abdominal ultrasonography. In 13 cases hepatic autoantibodies (ASMA, anti-LKM-1, LC-1, SLA-LP, AMA-M2) were analyzed, in 4 – magnetic resonance cholangiopancreatography and in 6 – liver biopsy were made.Results. Liver involvement were represented by an increase of liver enzymes in 58 (18.5%) cases. Chronic viral hepatitis C was diagnosed in 4 (1.3%) patients. Drug-induced hepatitis was found in 17 (5.4%) patients. Autoimmune liver diseases occured in 2 (0.6%) patients. In 2 (0.6%) patients, liver damage was associated with thrombotic microangiopathy (atypical hemolytic uremic syndrome, hereditary thrombophilia). In 15 (4.8%) cases, the most likely diagnosis was NAFLD. Lupus hepatitis was the most likely cause in 18 (5.7%) patients. Differential diagnosis in cases of liver involvement in patients with systemic lupus erythematosus requires assessment of risk factors for various liver diseases, age of the patients, level of liver enzymes, lupus activity, ultrasound signs of liver steatosis and secondary antiphospholipid syndrome.Determining the cause of the liver involvement for the patients with the systemic lupus erythematosus allows establishing better treatment tactic and improvement of the prognosis.

Hepatitis Lupus in Systemic Lupus Erythematosus in Male Patients

Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease characterized by autoantibodies against the cell nucleus and involves many organ systems in the body with unknown etiologies and various clinical manifestations, disease course and prognosis. SLE can be found at all ages, generally appearing at age 9-58 years with a peak at age 28 years. It is more common in women with a ratio of women to men 15: 1 to 22: 1. The highest incidence and prevalence of SLE was found in North America 23.2 / 100.000 population / year and 241 / 100.0000 population. In Indonesia, there has been an increase in visits to SLE patients from 17.9-27.2% in 2015 to 30.3-58% in 2017. One of the manifestations of SLE is hepatitis lupus, which is inflammation of the liver tissue. Lupus hepatitis can occur in 20-50% of patients with SLE. It was reported that a 20-year-old man presented with complaints of pain in the joints of the right and left hands which increased since 1 week. The patient also complained of reddish patches on the face, hair loss and mouth sores. Physical examination revealed anemic eye conjunctiva, malar rash, oral ulcer. During the joint examination, there was tenderness in bilateral MCP and PIP. The abdominal examination revealed hepatomegaly. Investigations revealed anemia, thrombocytopenia, increased liver function. Abdominal ultrasound revealed hepatomegaly. ANA profile examination was positive for anti RNP, anti-sm, and anti- ribosomal protein antibodies. The patient was diagnosed with Systemic Lupus Erythematosus with lupus hepatitis according to the ACR (American College of Rheumatology) criteria in which the patient had 6 criteria. The patient was given therapy with 2x125 mg of intravenous methyl prednisolone for 3 days and hydroxychloroquine 1x200 mg orally and other symptomatic drugs.